The retinal pigment epithelium (RPE) and choroid represent a differentiated system of the eye that sustains normal retinal health and function.
Best vitelliform macular dystrophy, known as Best disease, is an early-onset autosomal dominant condition in which accumulation of lipofuscin-like material within and beneath the RPE leads to progressive loss of central vision.
The lipofuscin-like material in the macular area appears as a yellow mass like the yolk of an egg that later becomes darker and irregular in color, a process known as “scrambling the egg”.
Best disease is frequently a reflection of mutations in the Bestrophin gene, which encodes a protein containing four putative transmembrane domains and localizes to the basolateral plasma membrane of RPE cells.
Human Bestrophin forms oligomeric chloride channels that are sensitive to intracellular calcium.
Missense mutations at the Bestrophin locus reduces or abolishes Bestrophin protein mediated membrane current.
The human Bestrophin gene encodes a 585 amino acid protein.
Applications:Suitable for use in Western Blot, Immunohistochemistry.
Other applications not tested.
Recommended Dilution:Western Blot: 1:500-1:2000Immunohistochemistry: 1:50-1:200Optimal dilutions to be determined by the researcher.
Storage and Stability:May be stored at 4°C for short-term only.
Aliquot to avoid repeated freezing and thawing.
Store at -20°C.
Aliquots are stable for 12 months after receipt.
For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
仕様
Size：100ul
Host：rabbit
Source Antibody：human
Grade：Affinity Purified
Purity：Purified by immunoaffinity chromatography.
Form：Supplied as a liquid PBS, 0.1% sodium azide, 50% glycerol.
Specificity：Recognizes endogenous levels of BEST1. Species Crossreactivity: Human
